The successful provision of optimal nutrition in children with serious diseases depends on the right diet, as well as the addition of specialized mixtures for its correction. The aim of the study was to study the effectiveness of supplemental nutrition in hereditary fermentopathy (cystic fibrosis) in children. The work included a three-year follow-up of 69 children aged 3 to 15 years suffering from cystic fibrosis. Patients were divided into two groups: 37 patients with cystic fibrosis group 1, receiving a modified version of treatment with additional enteral nutrition and 32 patients with cystic fibrosis group 2, receiving traditional treatment. The study conducted a comparative analysis of changes in trophic status in patients using nutritional support mixture produced in the Russian Federation – "Nutrien-standard"in diet therapy. Laboratory, functional and anthropometric parameters were monitored once a month. A significant increase in the physical development of patients was obtained only in the second year of use in the diet of supplementary nutrition, p<0.05. At the 3rd year of treatment, the results of positive dynamics of nutritional status in both groups were confirmed, while the increase in body mass index in the main group was 6.7%, and in the control group-only 1% (p<0.05). It is noted that the use of this mixture allows to achieve positive dynamics of nutritional status in 27% of patients and in 73% of cases to completely eliminate it. The obtained results prove the expediency of additional use of "Nutrient-standard" mixture in the complex therapy of patients with hereditary pancreatic enzyme deficiency, cystic fibrosis

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